The case of a 26-year-old male with sudden cardiac arrest due to ventricular fibrillation and the final diagnosis of arrhythmogenic right ventricular dysplasia-cardiomyopathy (ARVD/C) and initial early repolarization phenomenon is presented in detail. An additional analysis of early repolarization in additional 359 patients with ISFC/ESC diagnostic criteria of ARVD/C revealed a frequency within the threshold in healthy volunteers with 3% in isolated lateral leads and 7% in inferolateral leads. The high frequency of electrocardiographic early repolarization limited to inferior leads (22%) is probably due to late depolarization and represents an already reported typical feature of ARVD/C.